Born With a Small Right Ventricle: A Hypoplastic Right Heart Syndrome Story

Congenital Heart Defects or CHD’s for short, are the most common birth defect.  They occur in roughly 1 out of every 110 or 8 out of every 1,000 births. 

Congenital abnormalities are listed by the CDC as the most common cause of death in children under the age of one.  Of these abnormalities, heart defects are the most common. 

About 40,000 children are born every year with a heart defect, and of these 40,000 about a fourth have defects that would be considered serious enough to warrant surgery.  

Research is ongoing, but there are at least thirty five identified defects.  There are various causes of defects.  Some are caused by genetic or chromosomal abnormalities, some by medications the mother may have taken during pregnancy, or uncontrolled health issues she may have had, but more often than not, there is no known cause, and there is nothing the mother could have done to prevent it.  

I have an 11-year-old daughter who was diagnosed in utero with some of the most severe heart defects there are.  She has Pulmonary Atresia and Ebstein's anomaly which have led to her overall condition of Hypoplastic Right Heart Syndrome (HRHS).   
HRHS and its corresponding defect on the left side - HLHS are two of the most severe heart defects.

Hypoplastic means “small and undeveloped”, and in these defects, a whole side of the heart did not form correctly and is too small and undeveloped to do the job it needs to.  

With HRHS, the heart cannot pump blood to the lungs to receive oxygen, and with HLHS it cannot pump oxygenated blood to the body.  HRHS is known as a “single ventricle defect” as well as one of a group of defects called “cyanotic heart defects”.

Diagnosis before birth is done via a fetal echocardiogram.  If the ultrasound tech performing the standard 20 week ultrasound cannot visualize all four chambers, the mother will be referred for a more in depth ultrasound performed by a Perinatologist. 
That is what happened to me.  My OBGYN told me they didn't get a good look at my baby’s heart, and I would need to have a targeted ultrasound.  He reassured me it was likely nothing; as at this stage the baby was so small it could have just been facing the wrong way. 

While I was at the targeted ultrasound, I quickly realized there was something seriously wrong.  No one in the room was speaking to me, but I could hear them talking to each other about things like “lack of flow”, and things were “compensating” for something else.

I immediately knew something was wrong, because they wouldn't say things like that if everything was ok. 

Sure enough, a pediatric cardiologist came down and told me my baby did indeed have heart defects
, and I would have to travel to the Lower 48 to deliver her, as there were no surgeons in Alaska who performed cardiac surgery on infants and children.  
Treatment of HRHS defects is done with a set of three different surgeries, performed at different times.  

The first, the Blalock-Taussig Shunt, or BT shunt for short is done shortly after birth.  In some cases this is replaced by a PA Banding procedure.  This surgery places a shunt to increase blood flow to the lungs by connecting a branch of the subclavian or carotid artery to the pulmonary artery. 

 The second, the Bi-Directional Glenn is done around 6 months of age.  This surgery removes the first shunt and reroutes the superior vena cava to drain directly into the pulmonary artery.  This means the blood from the upper half of the body goes directly to the lungs for oxygen. 

The final surgery, the Fontan is done around age 3-4.  This completes the new circulation by connecting the inferior vena cava to the pulmonary artery so the blood from the entire body can go directly to the lungs for oxygen.  This essentially creates a new circulatory route and completely bypasses the right side of the heart.  
All of these are subject to each individual child’s diagnosis, physiology and a number of other factors.  We waited two weeks before they performed the first surgery.  Our surgeon told us this was done because after two weeks the pressures were better.  However, not every child can wait.  The timing of the second and third surgeries is not exact, but depends on a number of factors including the child's oxygen saturation levels, rate of growth, and how well they are thriving.  In general, they are done before the child enters Kindergarten.

There have been advancements in the surgical field, and some children do well enough, even with these severe defects to not need surgery right away.  Some don’t need the BT Shunt and can wait till the Glenn.  Some can even wait for the Fontan.

This can seem overwhelming but I have some good news! It gets better! I promise you.  
I found that the first two years or so were the most difficult due to all the doctor and therapy appointments.  After the age of 2 these decreased to the point I felt almost like I was parenting a normal child.  
The surgeries for these defects are by and large very successful, and the risk of death is quite small (10% or less).  It can still happen, but surgeons do a very good job keeping these kids alive.  More and more survive to adulthood and go on to get married, have jobs, etc.  In SOME instances women with heart defects can have children of their own, but this is something that would depend on the type and severity of the heart defect and would need to be monitored closely by their doctor. 

Not all doctors are well versed in heart defects, and have been known to unnecessarily frighten parents into thinking their child will not survive, or will have a poor quality of life.

If your doctor is doing this, GO FIND A NEW DOCTOR.  

Sometimes, yes, the defects would be severe enough that this might be true, but more often than not your child will be fine after some bumps in the road.

Today's guest poster is Laurel Warner.  She is a wife and mother of two beautiful girls! If you would like to connect with her you can contact her at 

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